Acute Basal Ganglia Infarction in Propionic Acidemia

An 8-year-old girl with propionic acidemia had acute and rapidly fatal symmetric necrosis of the caudate, globus pallidus, and putamen. Clinical presentation was with acute aphasia, generalized hypotonia, and muscle weakness. There was no evidence of metabolic decompensation, and analysis of the org...

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Bibliographic Details
Published in:Journal of child neurology 1995-01, Vol.10 (1), p.18-22
Main Authors: Haas, Richard H., Marsden, Deborah L., Capistrano-Estrada, Sylvia, Hamilton, Ronald, Grafe, Marjorie R., Wong, Wade, Nyhan, William L.
Format: Article
Language:English
Subjects:
Age of Onset
Aphasia
Basal Ganglia - blood supply
Caudate Nucleus - pathology
Cerebral Infarction - blood
Cerebral Infarction - diagnosis
Cerebral Infarction - pathology
Child
Fatal Outcome
Female
Globus Pallidus - pathology
Humans
Magnetic Resonance Imaging
Muscle Hypotonia
Necrosis - pathology
Propionates - blood
Putamen - pathology
Tomography, X-Ray Computed
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Summary:An 8-year-old girl with propionic acidemia had acute and rapidly fatal symmetric necrosis of the caudate, globus pallidus, and putamen. Clinical presentation was with acute aphasia, generalized hypotonia, and muscle weakness. There was no evidence of metabolic decompensation, and analysis of the organic acids of the urine indicated good metabolic control. Organic acids in the cerebrospinal fluid were unremarkable. These observations indicate that the pathophysiology of "metabolic stroke" is more complicated than previously thought. (J Child Neurol 1995;10:18-22).
ISSN:0883-0738
1708-8283
DOI:10.1177/088307389501000104